Study of the Relation Between Lipid Myocardial Overload Evaluated by Cardiac Magnetic Resonance Imaging (MRI), Alteration of Longitudinal Myocardial Deformations by Echocardiography, and Clinical Achievements (Functional, Biological and Electrical) in Fabry Disease, and Its Outcomes.

• Register: ClinicalTrials.gov
• Last refreshed on: 16 December 2017
• Main ID: NCT03123523
• Date of registration: 12/04/2017
• Prospective Registration: No
• Primary sponsor: University Hospital, Bordeaux
• Public title: Study of the Relation Between Lipid Myocardial Overload Evaluated by Cardiac Magnetic Resonance Imaging (MRI), Alteration of Longitudinal Myocardial Deformations by Echocardiography, and Clinical Achievements (Functional, Biological and Electrical) in Fabry Disease, and Its Outcomes. FABRY-Image
• Date of first enrolment: October 18, 2016
• Target sample size: 55
• Recruitment status: Recruiting
• URL: https://clinicaltrials.gov/show/NCT03123523
• Study type: Observational
• Phase: N/A

Countries of recruitment

France

Contacts

• Name: Réant Patricia, MD

• Name: Réant patricia, MD

Key inclusion & exclusion criteria

Inclusion Criteria:

Patients group :

- Adults (age =18 years), male and female.

- Patients diagnosed genetically having Fabry disease, with or without clinical cardiac
symptoms and with different evolution stades of the disease.

- For female in age of procreation, efficient contraception will be required and a
negative pregnancy test.

- Oral agreement of the patient after having read information note.

- Patient affiliated to social national Security registry.

Healthy volunteers group:

- Adults (age =18 years), male and female.

- Unscathed of cardiovascular pathologies and cardiovascular risk factors.

- For female in age of procreation, efficient contraception will be required and a
negative pregnancy test.

- Oral agreement of the patient after having read information note.

- Patient affiliated to social national Security registry.

Exclusion Criteria:

For the 2 groups :

- Extracardiac pathology limiting life expectancy <1 year (cancer).

- Pregnant or breastfeeding female.

- Claustrophobia.

- Mechanical prosthetic valve.

- Severe obesity > 140 kg

- Patients with intracardiac device (implantable cardiac defibrillator, pace maker,
resynchronisation), surgical clips not MRI compatible, neurosensorial stimulators,
cochlear implants, ferromagnetic foreign bodies (ocular, cerebral), neurosurgical
derivation valves)

- Impossibility to provide consent or refusal to sign the consent form.

For the patients:

- Previous history of hypersensitivity to gadolinium.



Age minimum: 18 Years
Age maximum: N/A
Gender: All

Health Condition(s) or Problem(s) studied

Fabry Disease

Intervention(s)

Biological: Biological assays

Diagnostic Test: Echocardiography at T0

Device: MRI with contrast agent injection

Device: MRI without contrast agent injection

Diagnostic Test: Exercise test

Diagnostic Test: Echocardiography at M24

Primary Outcome(s)

Cardiovascular symptoms [Time Frame: Baseline]

Metabolic exercise test marker : Expiratory volume / carbon dioxide production (VE/VCO2) [Time Frame: Baseline]

Metabolic exercise test marker : percentage of theoretical maximal heart rate [Time Frame: Baseline]

Metabolic exercise test marker : poor blood pressure adaptation to exercise [Time Frame: Baseline]

Metabolic exercise test marker: max level achieved [Time Frame: Baseline]

Metabolic exercise test marker : peak of Oxygen uptake (VO2) [Time Frame: Baseline]

Metabolic exercise test marker : percentage of expected peak VO2 [Time Frame: Baseline]

Biological marker : BNP elevation [Time Frame: Baseline]

Electrical markers at ECG and Holter ECG [Time Frame: Baseline]

Secondary ID(s)

CHUBX 2016/08

Source(s) of Monetary Support

Please refer to primary and secondary sponsors